Treatment with the mTOR inhibitor temsirolimus in patients with malignant PEComa

Italiano, Antoîne; Delcambre, Corinne; Hostein, Isabelle; Cazeau, Anne‐Laure; Marty, Marion; Avril, A.; Coindre, Jean‐Michel; Bui, B.

doi:10.1093/annonc/mdq044

Cited by 116 publications

(99 citation statements)

References 7 publications

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“…This approach is illustrated clearly by the relevant activity of imatinib in advanced dermatofibrosarcoma protuberans, 12,13 paclitaxel in angiosarcoma, 14,15 gemcitabine in leiomyosarcomas, 16,17 trabectedin in patients with myxoid/round cell liposarcomas, 18 and of mammalian target of rapamycin inhibitors in perivascular epithelioid cell tumors. 19 This evolution has been associated over the last 2 decades with the widespread use of additional lines of chemotherapy along with first-line anthracycline-based regimens; the increasing participation of patients with STS in investigational agents trials; the increased used of locoregional treatments for metastatic disease (surgery, radiofrequency ablation); and the general improvement in oncology supportive care.…”

Section: Discussionmentioning

confidence: 99%

Trends in survival for patients with metastatic soft‐tissue sarcoma

Italiano

Mathoulin‐Pélissier²,

Cesne

et al. 2010

Cancer

259

179

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BACKGROUND:The objective of this study was to determine whether the overall survival of patients with metastatic soft tissue sarcoma (STS) has improved over the last 20 years. METHODS: In total, 1024 patients who had synchronous metastatic ( RESULTS:The proportion of patients with SM, the interval between diagnosis and MM, and the clinical characteristics of the patients were similar across the 4 periods. Although there was no significant difference in the median overall survival (OS) from P1 through P2 (P1, 12.3 months; 95% confidence interval [CI], 9.9-14.7 months; P2, 11.4 months; 95% CI, 9-13.9 months), significant improvements were observed in the later periods (P3, 15 months; 95% CI, 11.8-18.2 months; P4, 18 months; 95% CI, 15.3-20.7 months; P ¼ .029; log-rank test). The 2-year OS rate also increased throughout the study period from 28.1% during P1 to 38.7% during P4. On multivariate analysis, period of diagnosis, age, histologic subtype, time to metastatic recurrence, French Federation of Cancer Centers Sarcoma Group grade, and the number of metastatic sites were independent prognostic factors for OS. CONCLUSIONS: The current analysis revealed that the median OS of patients with metastatic STS had improved by 50% during the last 20 years. These data should be considered in the interpretation of results from ongoing and future STS trials.

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Section: Discussionmentioning

confidence: 99%

Trends in survival for patients with metastatic soft‐tissue sarcoma

Italiano

Mathoulin‐Pélissier²,

Cesne

et al. 2010

Cancer

259

179

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“…Genetic analysis demonstrated that the mTOR pathway is activated in PEComas 9 and therefore mTOR inhibitors (such as temsirolimus and rapamycin) have been administered in clinical trials to patients at higher risk with encouraging results. 10 In our case, the diagnosis of PEComa avoided an improper indication to chemotherapy for recurrent GCT, which could be indicated based only on clinical findings. Surgical resection of the iliac mass seemed to be curative since the patient is disease-free after a follow-up of 38 months.…”

Section: Discussionmentioning

confidence: 72%

PEComa of soft tissues can mimick lymph node relapse in patients with history of testicular seminoma

Guglielmetti

Angelis

Mondino

et al. 2013

CUAJ

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Perivascular Epithelioid Cell tumour (PEComa) is rare. We describe a 39-year-old man who underwent a left radical orchidectomy and adjuvant radiation therapy for a stage IA classical testicular seminoma. He was diagnosed with a mass lateral to the right common iliac artery that was considered suspicious for late lymph node relapse after 3 years of follow-up. Due to the unusual location of the mass and the equivocal findings of percutaneous biopsy, a laparoscopic pelvic lymphadenectomy was performed. Final pathology revealed PEComa of soft tissue. The patient is diseasefree after 38 months of follow-up without adjuvant treatment. The presence of rare soft-tissue neoplasm should be considered in differential diagnosis of retroperitoneal masses during follow-up of germ cell tumours. Suspicious isolated recurrences of these neoplasms in unusual locations can require surgical excision to confirm diagnosis and avoid inappropriate treatment. IntroductionPEComas (Perivascular Epithelioid Cell tumors) are rare mesenchymal neoplasms characterized by a myomelanocytic phenotype and frequently unpredictable clinical behaviour. 1They can involve many anatomical sites and very few cases are reported worldwide.2 When PEComas are diagnosed in soft tissues, they can mimic enlarged lymph nodes and represent a possible confounder during follow-up of other solid tumours. Case reportWe present the case of a 42-year-old man with a history of left testicular pure seminoma. The patient was diagnosed with a palpable left testicular mass in October 2005. Scrotal ultrasound revealed a left hypoechoic testicular lesion, with normal controlateral testis. The serum markers were negative. No retroperitoneal lymphadenopathy or distant metastases were detected at chest x-ray (CXR) and abdominal contrast-enhanced computed tomography (CT). An uneventful left radical orchidectomy was performed. The pathology showed a classical seminoma with necrotic areas and invasion of the rete testis (pT1,N0,M0,S0; stage IA according to the 2009 TNM classification). After we consulted with the interdisciplinary team, the patient underwent adjuvant radiotherapy (25.2 Gy) on the left lombo-aortic lymph nodes.Follow-up included physical examination, serum markers every 4 months and CXR and abdominal CT scan every 6 months for the first 3 years, with negative results.At the abdominal CT scan performed September 2008, we found a single, enlarged 2.5-cm mass lateral to the right common iliac artery consistent with lymphadenopathy ( Fig. 1, part A, part B). The scrotal examination, the serum markers and the CXR were negative and the patient had no complaints. A positron emission tomography (PET) with fluorodeoxyglucose showed a hot spot (maximum standardized uptake value 3.76) in the area of the right common iliac lesion, which led us to a likely diagnosis of a late relapse of the testicular germ cell neoplasm. (Fig. 2) To confirm this diagnosis, a CT-guided fine needle aspiration biopsy was performed. (Fig. 1, part C). Cytology revealed epitheliomorphic cells ...

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“…Already numerous trials involving the PI3K-AktmTOR pathway [56][57][58][59][60][61][62][63] and mTOR inhibition [64][65][66][67][68][69] are on the way.…”

Section: Resultsmentioning

confidence: 99%